Long-term effects of cyclosporine A in Alport's syndrome
Long-term effects of cyclosporine A in Alport's syndrome
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DOI：
10.1046/j.1523-1755.1999.0550031051.x
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发表时间：
1999-03-01
影响因子：
19.6
通讯作者：
Nieto, J
中科院分区：
文献类型：
--
作者：
Callís, L;Vila, A;Nieto, J
背景。1991年，我们发表了8例阿尔波特综合征患者使用环孢素A （CsA）的初步结果。所有患者的蛋白尿明显减少或消失，对CsA的耐受性明显良好。这8名患者一直给予CsA治疗，目的是获得有关该疾病临床病程的进一步信息。这8例患者目前年龄在15 - 27岁之间，平均治疗时间为7 - 10年（x = 8.4年）。肾功能保持稳定，与csa治疗前相比，血清肌酐水平没有可评估的变化。所有患者的蛋白尿要么保持阴性，要么远低于预处理水平。所有患者在给予CsA 5个梨后进行了第二次肾活检。第一次活检未见病变加重或环孢素中毒的典型病变。在平均持续时间8.4年且肾功能未出现恶化的情况下，我们发现继续使用CsA治疗出现肾功能不全进展的Alport综合征患者可能有有益的效果。
Background. In 1991, our initial results of cyclosporine A (CsA) administration in eight patients with Alport's syndrome were published. A significant decrease in or disappearance of proteinuria and apparently good tolerance to CsA were observed in all patients.Methods. CsA administration has been maintained in these eight patients with the aim of obtaining further information about the clinical course of the disease. The ages of these eight patients currently range from 15 to 27 years, and the mean duration of treatment is from 7 to 10 years (x = 8.4 years).Results. Renal function has remained stable, with no evaluable changes in serum creatinine levels compared with pre-CsA treatment values. Proteinuria in all patients has either remained negative or are values far lower than pretreatment levels. A second renal biopsy was performed in all patients after five pears of CsA administration. No aggravation of the lesion present at the first biopsy or lesions typical of cyclosporine intoxication was observed.Conclusions. After a mean duration of 8.4 years and with no deterioration in renal function, we found possible beneficial effects of the continued treatment of CsA in patients with Alport's syndrome who present evidence of progression to renal insufficiency.